Statement of Research Significance
Research Question(s) or Topics:
There is a question regarding whether Developmental Gerstmann’s Syndrome (DGS) is a unique and valid diagnosis. There are very few case studies in the literature and most are in children. The present case report describes an undergraduate student who presented with symptoms of this syndrome. Main Findings: Through a comprehensive neuropsychological testing battery, we report evidence that this client experienced symptoms largely consistent with DGS, such as difficulties with math, confusion of her left and right sides, writing impairments, and an inability to name her fingers. However, assessment of this disorder posed challenges, largely due to the limited available literature and lack of a standardized testing battery. Study Contributions: This study identifies a possible case of a DGS in a well-functioning adult and presents the well-validated measures that were used and that may be included in a standardized testing battery in the future.
Introduction
Gerstmann’s Syndrome (GS) is a neurological disorder characterized by a classic tetrad of symptoms – an inability to name one’s own fingers (finger agnosia), deficits in mathematical abilities (acalculia), confusion of the left and right sides (right-left disorientation), and writing impairments (agraphia) – that are acquired though lesions to the left angular gyrus (Rusconi, Reference Rusconi2018). Developmental Gerstmann’s Syndrome (DGS) has been proposed as a related diagnosis to describe this same symptom tetrad that is present from childhood, with no clear etiology (Suresh & Sebastian, Reference Suresh and Sebastian2000). Proposed DGS cases have also shown deficits in motor coordination (constructional dyspraxia), leading this to be proposed as a fifth symptom of the disorder, although that status is debated (Miller & Hynd, Reference Miller and Hynd2004; Poeck & Orgass, Reference Poeck and Orgass1966).
The validity of both GS and DGS has been debated extensively in the literature, largely concerning whether these syndromes are distinct from other neurological disorders or, in the case of DGS, other learning disorders such as Williams syndrome, autism spectrum disorder, and nonverbal learning disability (Miller & Hynd, Reference Miller and Hynd2004; Rusconi, Reference Rusconi2018). There are limited DGS case studies and no epidemiologic studies, and the true incidence of DGS is unknown (Miller & Hynd, Reference Miller and Hynd2004). Cases demonstrating all four symptoms together have been rare (Ardila, Reference Ardila2014, Reference Ardila2020; Ardila & Rosselli, Reference Ardila and Rosselli2002; João et al., Reference João, Filgueiras, Mussi and Barros2017). Additionally, most DGS case studies report on children.
The case studies that do exist present strong evidence for DGS. Many of these cases exhibited neurological abnormalities including abnormal EEG readings, seizures, or recurrent headaches (Suresh & Sebastian, Reference Suresh and Sebastian2000, 10 cases); fragile X syndrome (Grigsby & Hagerman, Reference Grigsby and Hagerman1989); systemic lupus erythematosus (Jung et al., Reference Jung, Yeo, Sibbitt, Ford, Hart and Brooks2001); multiple sclerosis (Ehrlé et al., Reference Ehrlé, Maarouf, Chaunu, Sabbagh-Peignot and Bakchine2012), and cerebellar neoplasia (Werneck, Reference Werneck1975), though cases have also been reported without neurological or psychiatric abnormalities (Benson & Geschwind, Reference Benson and Geschwind1970). PeBenito (Reference PeBenito1987) presented ten child cases and reviewed literature reflecting a variable presence of brain abnormalities in DGS.
We found only three case studies of DGS diagnosed in adults (Chang et al., Reference Chang, Hsu, Hsiao, Chang and Tzeng2014, age 23; Pirozzolo & Rayner, Reference Pirozzolo and Rayner1978, age 22; Tzeng et al., Reference Tzeng, Hu, Cheng and Yeh2009, age 20) and one adult case of GS where a developmental origin was suspected (Ehrlé et al., Reference Ehrlé, Maarouf, Chaunu, Sabbagh-Peignot and Bakchine2012, age 42). The current case study aims to add to the existing literature by suggesting the presence of the GS tetrad, along with constructional deficits, in an adult with no clear history of neurological damage.
Case presentation
Presenting problem
The client was a 20-year-old, right-handed, White cisgender female undergraduate student who self-referred to the Boston University Center for Anxiety and Related Disorders for a neuropsychological evaluation to seek academic accommodations for math classes. She struggled to understand content in a required statistics course that she failed twice. She reported a longstanding history of difficulties with math courses, with a particular weakness in both understanding and solving word problems. The client reported receiving grades in the A to B range in her math courses before college, owing largely to her teachers’ informal provision of extra time on tests.
The client signed a Consent for Neuropsychological Assessment that was approved by the Boston University Charles River Campus Institutional Review Board for her data to be used in archival research, and consented to publication of her data. This research was completed in accordance with the Helsinki Declaration.
Medical and psychiatric history
The client reported multiple medical problems, primarily an autoimmune disorder. Her doctors could not identify a specific diagnosis, but treated the condition like lupus. She also had polycystic ovary syndrome (PCOS) and alopecia, both related to her autoimmune disorder. She was prediabetic and insulin resistant, though these were unrelated to her autoimmune disorder. She reported prescriptions of sertraline, metformin, doxycycline, citalopram, lansoprazole, montelukast, fluticasone furoate, fluticasone propionate, iron, vitamin D, and fexofenadine hydrochloride. Dosages were not available, except that sertraline was at the lowest dose typically prescribed.
The client reported no known developmental issues or birth complications and was the oldest of two children in her family. She had no history of head injury. She had a brain scan before age six. Neither she nor her mother recalled why she had the scan besides her frequent headaches, but thought the results were normal.
Prior to entering school, she had severe sensitivity to external stimuli. She was afraid to leave the house, as she perceived other people as moving too fast, and she found all noises to be too loud. An evaluation found negative results for autism and other abnormal developmental concerns, though she continued to be sensitive to sounds and light. She reported often listening to white noise while studying and being easily distracted by sounds during tests. During our evaluation, she became distracted by a ticking clock in the testing room.
The client reported math anxiety. During math lectures, she would spend excessive time trying to understand one concept and would then miss later presented material. She reported anxiety in general manifesting as difficulty relaxing and feeling overwhelmed. As a child, she received diagnoses of vomit phobia and obsessive-compulsive disorder. Her symptoms improved after therapy and martial arts training, remitting around age eight. She also experienced psychological and sexual abuse during high school, and as a result developed symptoms of posttraumatic stress disorder and major depressive disorder. She reported no alcohol or substance use in her life.
The client reported no significant social conflicts; however, she described herself as “not the most social.” When she was younger, she would choose martial arts practice over social interaction. She was in a supportive relationship with her girlfriend and had friends. She initially appeared somewhat guarded, with constricted affect; however, she became more open with the examiner over time and was pleasant to work with.
Evidence for DGS
The client received a comprehensive neuropsychological assessment. See Table 1 for a list of the tests, her standard scores, and descriptive labels for scores less than average. Test scores are described in accordance with guidelines from the American Academy of Clinical Neuropsychology consensus conference (Guilmette et al., Reference Guilmette, Sweet, Hebben, Koltai, Mahone, Spiegler, Stucky and Westerveld2020). See Table 2 for information on labels used in Table 1. Her test performance and difficulties with daily functioning are presented below in relation to the DGS symptom categories.
Neuropsychological test results
WAIS-IV = Wechsler Adult Intelligence Scale-IV; WJ-IV = Woodcock-Johnson IV Tests of Achievement; CTOPP = Comprehensive Test of Phonological Processing (Wagner et al., Reference Wagner, Torgesen, Rashotte and Pearson1999); CPT 3 = Conners Continuous Performance Test 3; D-KEFS = Delis-Kaplan Executive Function System Test, TMT = Trail Making Test, TT = Tower Test; CVLT-3 = California Verbal Learning Test II; WMS-IV = Wechsler Memory Scale-IV; Benton JOL = Benton Judgment of Line Orientation Test; ASRS = Adult ADHD Self-Report Scale.
* Reflects descriptive labels as shown in Table 2. Scores that represent average or above performance are not indicated in the final three columns. Scores in the low average range indicate performances that may or may not represent challenges and should be interpreted within the context of the client’s broader functioning.
Descriptive labels and standard score ranges
Test score labels are in accordance with the guidelines from Guilmette et al. (Reference Guilmette, Sweet, Hebben, Koltai, Mahone, Spiegler, Stucky and Westerveld2020) and listed with corresponding values of various standard scores generated by the test battery.
Dyscalculia
The client reported a history of difficulties with math. Specifically, she struggled in understanding abstract concepts, particularly in trigonometry. Her struggle to understand word problems contributed to her running out of time on exams. She found calculating fractions and percentages challenging and required drawing a pie chart before making calculations. She had difficulty rounding numbers and thus always rounded up. Her difficulty with numerical reasoning mildly affected her functioning at work. She had trouble remembering the numerical code to the locker room, and instead memorized the movements to enter the code rather than the numbers. She had difficulty with counting and could not accurately count the money in the cash drawer at the end of her shift; as a result, she was exempt from this task.
Within this context of functional impairment, the client performed in the low average to below average range on working memory tests involving recalling and manipulating numbers in the Wechsler Adult Intelligence Scale 4th Edition (WAIS-IV;Wechsler, Reference Wechsler2014) (Digit Span, low average; Arithmetic, below average; Working Memory Index, below average). In contrast, she performed average or above average on the mathematical achievement tests of the Woodcock-Johnson Tests of Achievement-Fourth Version (WJ-IV; Schrank & Wendling, Reference Schrank and Wendling2018).
Right-left disorientation
The client reported a history of substantial right-left disorientation. In elementary school, she was almost held back a year due to difficulty distinguishing her left and right hands and tying her shoes. She struggled to remember sequences of martial arts movements that alternated between left and right. Instead, she learned the sequences using muscle memory, similar to her method of memorizing her locker code. In her undergraduate statistics course, she had difficulty understanding the normal curve as she could not remember whether “above average” and “below average” were located to the right or left of the mean. Although she reported no difficulties with telling time or reading a map, she was 30 minutes late to two appointments due to confusion about time and location.
She was administered the Benton Right-Left Orientation test (Benton et al., Reference Benton, Hamsher and Varney1983) ) to assess these difficulties. This untimed test requires pointing to lateral body parts on verbal command. The client scored within the average range, but provided answers slowly and after much consideration. She also performed accurately but slowly on the Delis-Kaplan Executive Function System Test (D-KEFS) Tower Test, which involves spatial reasoning, planning, and an understanding of whether to place discs on pegs to the left or right (Delis et al., Reference Delis, Kaplan and Kramer2001). Her Time per Move Ratio was in the low average range, and her Mean First Move Time was in the exceptionally low range. However, she made no rule violations and her total achievement and accuracy scores were within the average range.
Dysgraphia
The client reported difficulties learning to write in elementary school. Instead of writing horizontally, she would write each individual word on a separate line vertically. She received a full year of tutoring to correct this pattern. She completed the Cookie Theft Picture test from the Boston Diagnostic Aphasia Examination (Kaplan, Reference Kaplan1983) and the WJ-IV Sentence Writing Fluency test. The former test provides information on narrative language and writing skills and is untimed. In both writing samples, the client inappropriately capitalized letters in the middle and at the ends of words. Additionally, in her narrative writing sample, she demonstrated punctuation errors and wrote each individual sentence on a separate line, similar to her report of writing individual words on separatelines as a child (Figure 1).
A writing sample from the client’s neuropsychological assessment battery. The image displays the client’s written description of the Boston Cookie Theft Picture.
Finger agnosia
The Benton Finger Localization test was administered to assess the client’s ability to recognize her own fingers in an untimed fashion (Benton et al., Reference Benton, Hamsher and Varney1983). She provided answers slowly and with much consideration. Her right hand performance was average while her left hand performance was low average. She could only name two fingers.
Constructional apraxia
The client performed low average on tests of spatial reasoning including the WAIS-IV Perceptual Reasoning Index, as well as the individual subtests of Visual Puzzles and Block Design, the latter of which involves a motor component. Without time limitations, her Block Design score rose to average. Given these challenges and the above noted difficulties with writing horizontally, she was administered a measure of visual-motor coordination (Beery Visual-Motor Integration Test: Beery VMI; Beery & Beery, Reference Beery and Beery2006) and performed below average. She was not given the Beery VMI Visual Perception or Motor Coordination conditions, although her score on the Benton Judgment of Line Orientation (JoL) Test (Benton et al., Reference Benton, Hamsher and Varney1983)) demonstrated average visual perception. On the Grooved Pegboard Test (Matthews & Klove, Reference Matthews and Klove1964) of motor speed and dexterity, she performed exceptionally low with her dominant hand, and low average with her nondominant hand.
In contrast to the Beery VMI, she performed with average accuracy in her copy of the Rey-Osterreith Complex Figure Test (Rey; Osterrieth, Reference Osterrieth1944; Rey, Reference Rey1941). While her organization was high average (88th percentile), she performed in the low average range in her immediate recall of the design. However, she performed average on the delayed recall.
Other areas of weakness
On the Finger Tapping Test (Reitan & Wolfson, Reference Reitan1985), the client’s simple motor speed was exceptionally low for both hands and highly variable (e.g., right hand range of 17 – 33). A second administration, though improved, was still exceptionally low. When queried, she reported being unaware of her inconsistent and low performance. Nevertheless, she scored in the average range on the motor speed component of the D-KEFS Trail Making Test. The client also performed low or below average on two language-related tests (see Table 1), though her low scores may have been due to chance.
Areas of strength
The client performed well at school in all classes except math, receiving mostly A’s while also working 10 hours per week. She reported strengths in English, history, and science.
She demonstrated average to above-average performance on tests of reading, writing, language, attention, and executive functioning with a few exceptions as noted above (see Table 1). Her performance on tests of visual and verbal memory were similarly average or high average, except for her Rey immediate recall score.
The client completed a questionnaire screen for symptoms of attention deficit hyperactivity disorder (ADHD), the Adult ADHD Self-Report Scale (Kessler et al., Reference Kessler, Adler, Ames, Demler, Faraone, Hiripi, Spencer, Ustun and Walters2005). She reported only two symptoms at a clinical level (fidgeting or squirming and feeling overly active); thus, further evaluation of ADHD was not warranted. Her self-reported attentional difficulties (i.e., getting easily distracted during tests) were not likely due to ADHD.
Discussion
This case report describes a client who sought academic accommodations for math, but upon evaluation, demonstrated a broader range of deficits, including right-left disorientation, mild finger agnosia, idiosyncrasies in writing, and mild constructional deficits. She also demonstrated strengths in tests of reading, writing, and memory. Her symptoms were similar to previous discussions of DGS, though not as severe as previously reported cases. She was diagnosed with Other Specified Neurodevelopmental Disorder; recommendations included academic accommodations for statistics courses and specialized tutoring.
The client demonstrated motor speed and dexterity performance that was slow and variable. It has been proposed that difficulties with fine motor movement are connected to finger agnosia, as well as to development of counting and calculation abilities, in children (Fischer et al., Reference Fischer, Suggate and Stoeger2022). The client was also slow on the Tower, Right-Left Orientation, and Finger Localization tests, all involving some motor involvement, and on the Stroop Color and Word test (Stroop, Reference Stroop1935), which did not involve a motor component.
The client’s performance on WAIS-IV Arithmetic was notably lower that her WJ-IV math tests. One possible explanation is that the WJ-IV tests are completed on paper while the WAIS-IV test requires examinees to perform calculations using working memory. It is also possible that the questions asked on the WAIS-IV test involved processes that were more difficult for the client to process. While the client clearly struggled with some mathematical concepts, her scores were not clear evidence of dyscalculia.
There was also a disparity between the client’s Rey and Beery VMI scores with worse performance on the latter test. Both tests assess visual construction ability, but differ in other respects. The Beery VMI consists of simple figures that become increasingly complex, and evaluates test performance in terms of developmental level of the integration of visual perception and finger/hand movements, while the Rey measures visual organization, motor planning skills, and visual memory in addition to visual construction skills. In a study in children, the percent of variance accounted for in the two tests was found to never be above 31%, suggesting that they do not overlap completely (Demsky et al., Reference Demsky, Carone, Burns and Sellers2000).
The Beery VMI may have provided additional challenges to the client in that the figures may require higher levels of motor coordination and manual dexterity to copy accurately (e.g., rounded edges vs. straight lines), and involve understanding depth of images (e.g., overlapping solid lines and shapes). The client demonstrated generally good executive skills and good organizational abilities on the Rey, which may have helped her compensate for visual construction weaknesses.
There have been very few reported cases describing DGS in adults. One case study describes a 22-year-old female with deficits in finger recognition and right-left orientation, as well as abnormal inverted writing patterns, but does not report on measures of math abilities (Pirozzolo & Rayner, Reference Pirozzolo and Rayner1978). The authors determined that, similar to the present case, the client’s writing difficulties may have been due to abnormal spatial orientation. Another adult DGS case describes a 20-year-old male inpatient soldier in Taiwan who presented with major depressive disorder (Tzeng et al., Reference Tzeng, Hu, Cheng and Yeh2009). Neuropsychological testing revealed deficits in reading and writing, mental calculations, right-left orientation, and finger recognition, and his composite WAIS-R scores were exceptionally low. A third case report describes a 23-year-old male Taiwanese soldier who presented with all four DGS symptoms and had learned strategies to compensate for deficits (Chang et al., Reference Chang, Hsu, Hsiao, Chang and Tzeng2014).
A notable commonality in two of these cases is that although these clients were able to write fluently, they all exhibited spatial difficulties with writing. In the case of Pirozzolo & Rayner (Reference Pirozzolo and Rayner1978), the client wrote upside down from right to left, while Tzeng et al.’s (Reference Tzeng, Hu, Cheng and Yeh2009) client wrote some Chinese characters reversed from right to left. In the present case, our client demonstrated difficulties with writing continuous lines horizontally. Additionally, none of these cases had a clear neurological cause. A limitation of the present case report is that we did not have neurological testing available.
Another possible explanation for a deviation from other case studies is lack of consistency in assessments previously used to diagnose GS and DGS (Miller & Hynd, Reference Miller and Hynd2004; Rusconi, Reference Rusconi2018). Because there is no standard set of assessments used to diagnose these disorders, many previously reported cases used assessments that are either not standardized or have been deemed invalid by others in the field (Miller & Hynd, Reference Miller and Hynd2004; Rusconi, Reference Rusconi2018). The present case used well-validated assessments. Future diagnostic tests would benefit from a fully standardized testing battery for assessing DGS (Rusconi, Reference Rusconi2018).
The client’s symptoms may have been related to her autoimmune disorder. One previous GS case describes the development of symptoms over time alongside lupus-induced neural atrophy (Jung et al., Reference Jung, Yeo, Sibbitt, Ford, Hart and Brooks2001). Decreased manual dexterity has also been documented in individuals with lupus, including decreased speed on the finger tapping test (Denburg et al., Reference Denburg, Carbotte, Ginsberg and Denburg1997; Johnsson et al., Reference Johnsson, Sandqvist, Bengtsson and Nived2008). Some cognitive deficits have also been reported in association with lupus, such as verbal and nonverbal memory (Emori et al., Reference Emori, Matsushima, Aihara, Ohta, Koike, Miyasaka and Kato2005; Stojanovich et al., Reference Stojanovich, Zandman-Goddard, Pavlovich and Sikanich2007). However, these deficits do not map fully onto the symptoms of our client, and she reported that her autoimmune disorder was not in fact lupus. Further, her symptoms were present in childhood and had not worsened over time.
The client also took nine different medications daily. These medications are not known to affect cognitive function or psychomotor speed in isolation (Enderami et al., Reference Enderami, Shariati, Zarghami, Aliasgharian, Ghazaiean and Darvishi-Khezri2025; Hegerl et al., Reference Hegerl, Mergl, Henkel, Pogarell, Müller-Siecheneder, Frodl and Juckel2005; Paul et al., Reference Paul, Gray and Lange2002; Paul et al., Reference Paul, Gray, Love and Lange2007; Volz & Sturm, Reference Volz and Sturm1995); in fact, evidence suggests sertraline and citalopram may reduce motor slowing associated with depressive symptoms (Khan et al., Reference Khan, Mehta, Kade and Turankar2011; Roy et al., Reference Roy, Harvey, Berlim, Mamdani, Beaulieu, Turecki and Lepage2010; Schrijvers et al., Reference Schrijvers, Maas, Pier, Madani, Hulstijn and Sabbe2009). However, the combination of her medications could have caused memory- and motor-related problems that have not yet been demonstrated in a controlled empirical study.
The client’s psychiatric history is also notable. She reported high anxiety around tests, as well as significant trauma history. Symptoms related to anxiety and posttraumatic stress disorder (e.g., difficulty with concentration, hypervigilance) may have contributed to low processing speed during examinations. However, reactions to trauma were unlikely to explain all her symptoms as she reported that her cognitive difficulties were present in early childhood.
Other potential explanations for the client’s low performance include a possible response style of emphasizing accuracy over speed. However, this response style was only observed on the tests related to the four DGS symptoms. The client’s motor difficulties could have been related to developmental coordination disorder; however, she did not report difficulties with coordination in her daily life. Visuospatial deficits and dyscalculia are often included as symptoms of nonverbal learning disability (Fisher et al., Reference Fisher, Reyes-Portillo, Riddle and Litwin2022). Aspects of her functioning are consistent with a math disorder, but her profile is not fully consistent with this disorder. Due to the high number of tests administered, her low scores could have been due to chance. Although an unrecognized brain lesion could have contributed to her symptoms, she did not have notable results from her brain scan as a child, and she denied any potential causes of lesions beyond her autoimmune disorder.
Similar to many other case studies, our client’s presentation did not neatly fit the tetrad of GS symptoms. For example, despite difficulties with completing the tests, she largely performed within the average range for finger localization, right-left orientation, and various math tests. However, she had received specialized tutoring and developed compensatory strategies that may have allowed her high level of functioning, such as using “muscle memory” to remember spatial sequences involving right-left orientation (i.e., martial arts sequences and locker combinations). The literature suggests that individuals with DGS who receive remedial education can improve their academic achievement and potentially their cognitive ability (Gracia-Bafalluy & Noël, Reference Gracia-Bafalluy and Noël2008; PeBenito, Reference PeBenito1987), which may be the case for the present client.
Acknowledgements
The authors have no conflicts of interest to disclose. This evaluation was conducted at the Center for Anxiety and Related Disorders, a training clinic affiliated with the doctoral program in Clinical Psychology at Boston University. Preparation of this manuscript was supported by the Sierra Pacific Mental Illness Research Education and Clinical Centers (MIRECC) Fellowship.
Competing interests
The authors declare none.
